离子通道
溶酶体
细胞生物学
细胞内
化学
细胞器
细胞外
离子运输机
膜电位
生物物理学
生物
生物化学
膜
受体
酶
作者
Andra M. Sterea,Shekoufeh Almasi,Yassine El Hiani
出处
期刊:Cell Calcium
[Elsevier BV]
日期:2018-03-18
卷期号:72: 91-103
被引量:51
标识
DOI:10.1016/j.ceca.2018.02.006
摘要
Lysosomes serve as the control centre for cellular clearance. These membrane-bound organelles receive biomolecules destined for degradation from intracellular and extracellular pathways; thus, facilitating the production of energy and shaping the fate of the cell. At the base of their functionality are the lysosomal ion channels which mediate the function of the lysosome through the modulation of ion influx and efflux. Ion channels form pores in the membrane of lysosomes and allow the passage of ions, a seemingly simple task which harbours the potential of overthrowing the cell's stability. Considered the master regulators of ion homeostasis, these integral membrane proteins enable the proper operation of the lysosome. Defects in the structure or function of these ion channels lead to the development of lysosomal storage diseases, neurodegenerative diseases and cancer. Although more than 50 years have passed since their discovery, lysosomes are not yet fully understood, with their ion channels being even less well characterized. However, significant improvements have been made in the development of drugs targeted against these ion channels as a means of combating diseases. In this review, we will examine how Ca2+, K+, Na+ and Cl- ion channels affect the function of the lysosome, their involvement in hereditary and spontaneous diseases, and current ion channel-based therapies.
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